Abstract

Background

Hemifacial atrophy (Parry-Romberg syndrome) is a relatively rare disease. The etiology
of the disease is not clear. Some authors postulate its relation with limited scleroderma
linearis. Linear scleroderma "en coup de sabre" is characterized by clinical presence
of most commonly one-sided linear syndrome. In a number of patients, neurological
affection is the medium of the disease. The treatment of both scleroderma varieties
is similar to the treatment of limited systemic sclerosis.

Case presentation

We present two cases of a disease: a case of a 49-year-old woman with a typical image
of hemifacial atrophy, without any changes of the nervous system and a case of a 33-year-old
patient with an "en coup de sabre" scleroderma and with CNS tumor.

Conclusion

We described typical cases of a rare diseases, hemifacial atrophy and "en coup de
sabre" scleroderma. In the patient diagnosed with Parry-Romberg syndrome, with Borrelia
burgdoferi infection and with minor neurological symptoms, despite a four-year case
history, there was a lack of proper diagnosis and treatment.

In the second patient only skin changes without any neurological symptoms could be
observed and only a precise neurological diagnosis revealed the presence of CNS tumor.